Abstract
Background: Sickle cell disease (SCD) is an autosomal recessive disease caused by a single gene mutation, leading to sickle-shaped red blood cells, causing many clinical complications. Resulting complications may affect the growth of the SCD patients that is a strong measure of severity of disease and helps in disease management strategies in any area. Eastern province of Saudi Arabia has one of the highest SCD incidences. Nevertheless, no studies have been previously carried out of about clinical outcome of SCD in Al-Ahsa area of eastern province. Therefore, this study was conducted to find out the impact of SCD children at king Abdulaziz Hospital Al-Ahsa.
Methods: All pediatric SCD patients were included in the study. Patient data was taken from hospital information system and analyzed using SPSS version 27.
Results: A total of 53 patients were studied. The male to female ratio was 1.4:1 and mean age was 3.3 years (range: 1-9). Eighteen (34%) did not present with sickle cell crisis possibly due to ameliorating effects of high HbF and G6PD deficiency. Although growth parameters of SCD patients were not statistically different from international standards, there was significant difference between weight of SCD patients in recurrent sickle cell crisis group and non-crisis sickle cell (NC-SC) group at diagnosis and after clinical interventions (p= 0.04 and 0.03, respectively) that included hydroxyurea. The corrected reticulocyte (at diagnosis and after clinical intervention) and WBC counts were statistically significant between hydroxyurea and non-hydroxyurea groups (p-value < 0.05).
Conclusions: Overall, one-third of SCD patients in Al-Ahsa region have mild disease and hydroxyurea can minimize the SCD severity through lowering corrected reticulocyte and WBC counts. Exact mechanisms of mild SCD and hydroxyurea in minimizing disease severity are needed to be elucidated.